Neupsy Key

Essential Tremor Chronic, progressive neurologic condition; 4 to 12 Hz tremor may involve arms, neck, voice, chin. Tremor primarily with movement (kinetic) or sustained posture. Rarely present at rest, in…

Dystonia Sustained muscle contractions, frequently cause twisting repetitive movements or abnormal postures (see also Chapter 9). Also called “torsion dystonia.” Movements sometimes rhythmic (dystonic tremor). EMG in dystonia: simultaneous contraction…

Gilles De La Tourette Syndrome Most common cause of tics. Diagnostic criteria: multiple motor and phonic tics; change in character over time; onset before age 21 years; symptoms wax and…

Myoclonus Brief, “lightning-like” muscle jerks with typically brief EMG bursts (20 to 100 msec). “Negative” myoclonus: sudden, brief lapse of contraction (e.g., asterixis). Prevalence 8.6/100,000. Differential diagnosis: no other movement…

Sydenham and Other Forms of Chorea Most common causes of chorea: Tables 110.1, 110.2. Table 110.1 Common Causes of Chorea Hereditary Huntington disease Huntington disease-like syndromes (HDL1, HDL2, HDL3) Hereditary…

Huntington Disease Progressive; autosomal dominant; usually adult onset; with movement disorder (usually chorea), personality disorder, dementia. Pathology: generalized brain atrophy; striatum (caudate nucleus, putamen) most affected. Biochemistry: loss of striatal…

Hereditary Ataxias See Table 108.1 for list of disorders; Tables 108.2, 108.3 for clinical features and inheritance. Notes about selected conditions follow. Table 108.1 Hereditary Ataxias by Mode of Inheritance…

Alzheimer Disease and Other Dementias Definition of dementia (according to DSM-IV): “Multiple cognitive deficits that are sufficiently severe to cause impairment in occupational or social functioning.” Must involve memory plus…

Acquired Neuropathies Guillain-Barré Syndrome (GBS) and Variants Also “acute inflammatory demyelinating polyneuropathy.” Acute onset of peripheral and cranial nerve dysfunction. Frequently preceded by respiratory or gastrointestinal infection immunization, or surgery….

Hereditary Neuropathies Selected inherited neuropathies and their features listed in Table 105.1. Clinical classification in Table 105.2. Table 105.1 Inherited Neuropathies Disorder Locus/gene Inheritance Protein Testing method CMT1A 17p11.2/PMP22 AD…